Trial reportJournal of neurosurgery2014
Prospective natural history study of central nervous system hemangioblastomas in von Hippel-Lindau disease.
Trial report in Journal of neurosurgery, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 89 papers, 2 of them syntheses that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
89 citing papers in PubMed, 2 syntheses or guidelines pooled it, 201 citations in OpenAlex.
- Stereotactic radiosurgery in the management of central nervous system hemangioblastomas: a systematic review and meta-analysis.Neurosurgical review · 2025Pooled it
- Stereotactic radiosurgery for central nervous system hemangioblastoma: systematic review and meta-analysis.Journal of neuro-oncology · 2018Pooled it
- Belzutifan for patients with von Hippel-Lindau disease-associated CNS haemangioblastomas (LITESPARK-004): a multicentre, single-arm, phase 2 study.The Lancet. Oncology · 2024Trial
- Growth rate and fate of untreated hemangioblastomas: clinical assessment of the experience of a single institution.Journal of neuro-oncology · 2019Trial
- Gamma Knife Stereotactic Radiosurgery favorably changes the clinical course of hemangioblastoma growth in von Hippel-Lindau and sporadic patients.Journal of neuro-oncology · 2019Trial
- Imaging diagnosis of cystic intraparenchymal brain neoplasms.Japanese journal of radiology · 2026Review
- Cyst wall enhancement is associated with local recurrence after resection of intracranial hemangioblastoma: an exploratory retrospective cohort study.Journal of neuro-oncology · 2026Article
- Somatic mutational landscape in von Hippel-Lindau familial hemangioblastoma.Molecular oncology · 2026Article
- Cancer predisposition syndromes: an imaging review.Cancer imaging : the official publication of the International Cancer Imaging Society · 2026Review
- Belzutifan-induced tumor regression in sporadic hemangioblastoma: a case report and literature review.Journal of neuro-oncology · 2026Review
- Neurogenetic tumor syndromes: The current landscape of workup and treatment.Neuro-oncology practice · 2026Review
- Von Hippel-Lindau syndrome presenting as a lateral ventricular space-occupying lesion: a case report.Frontiers in oncology · 2026Article
- Clinical features and long-term surgical outcomes of conus medullaris hemangioblastomas.Egyptian journal of neurosurgery · 2026Article
- Safety and effectiveness of stereotactic radiosurgery for larger hemangioblastomas (>2cc): A multi-center retrospective study.PloS one · 2026Article
- Von Hippel-Lindau Disease-Associated Endolymphatic Sac Tumours: Seven Cases and Genotype-Phenotype Features.Current oncology (Toronto, Ont.) · 2025Article
- Familial Von Hippel-Lindau Disease: A Case Series of Cerebral Hemangioblastomas with MRI, Histopathological, and Genetic Correlations.Life (Basel, Switzerland) · 2025Article
- Canine Hemangioblastoma: Case Series and Literature Review.Animals : an open access journal from MDPI · 2025Article
- Gross total resection is associated with improvement and prognosis even in von Hippel-Lindau disease-related hemangioblastomas: Nationwide registry in Japan.Neuro-oncology practice · 2025Article
- Pediatric and adolescent von Hippel-Lindau disease: tumor profiles, genotype-phenotype correlation and comparison with adults.Journal of endocrinological investigation · 2025Article
- Dual Inhibition of HIF-1α and HIF-2α as a Promising Treatment for VHL-Associated Hemangioblastomas: A Pilot Study Using Patient-Derived Primary Cell Cultures.Biomedicines · 2025Article
29 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
10 authors at 5 institutions in 1 country.
Funding
Abstract
objectThe tumors most frequently associated with von Hippel-Lindau (VHL) disease are hemangioblastomas. While they are associated with significant neurological impairment and mortality, their natural history and optimal management have not been fully defined.
methodsPatients with VHL were enrolled in a prospective study designed to define the natural history of CNS hemangioblastomas. In the present analysis, serial imaging, laboratory, genetic, and clinical data were evaluated in those with at least 2 years of follow-up data.
resultsAt study entrance 225 patients (111 males, 114 females) harbored 1921 CNS hemangioblastomas in the supratentorial compartment (21 tumors [1%]), cerebellum (865 [45%]), brainstem (129 [7%]), spinal cord (689 [36%]), cauda equina (212 [11%]), and nerve roots (5 [0.3%]; follow-up 15,819 hemangioblastoma-years). Increased tumor burden was associated with partial deletions in the VHL gene (p = 0.005) and male sex (p = 0.002). Hemangioblastoma development (median 0.3 new tumors/year) was associated with younger age (p < 0.0001) and more tumors at study entrance (p < 0.0001). While 1278 hemangioblastomas (51%) did not grow, 1227 hemangioblastomas (49%) grew in a saltatory (886 [72%]), linear (76 [6%]), or exponential (264 [22%]) pattern. Faster tumor growth was associated with male sex (p = 0.001), symptomatic tumors (p < 0.0001), and tumors associated with cysts (p < 0.0001). Location-dependent tumor size was the primary predictor of eventual symptom formation (159 symptomatic tumors [6.3%]; area under the curve > 0.9).
conclusionsCentral nervous system hemangioblastoma burden in VHL is associated with partial germline deletions and male sex. Unpredictable growth of hemangioblastomas compromises assessment of nonsurgical therapies. The judicious treatment of symptom-producing hemangioblastomas, while avoiding unnecessary treatment of asymptomatic tumors that may not progress, can provide clinical stability.
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.